Right Heart Involvement in Patients with Sarcoidosis

Abstract

The left ventricle (LV) is affected in 20-25% of patients with sarcoidosis and its involvement is associated with morbidity and mortality. However, effects of sarcoidosis on the right ventricle (RV) are not well documented. Our aims were to investigate the prevalence of RV dysfunction in patients with sarcoidosis and determine whether it is predominantly associated with direct cardiac involvement, severity of lung disease, or pulmonary hypertension (PH). We identified 50 patients with biopsy-proven extra-cardiac sarcoidosis and preserved LV function, who underwent echocardiography, pulmonary function (PF) testing, and cardiovascular magnetic resonance. RV function was quantified by free wall longitudinal strain. Tricuspid valve Doppler and estimated right atrial pressure were used to estimate systolic pulmonary artery pressure. Myocardial late gadolinium enhancement was considered diagnostic for cardiac sarcoidosis and assumed to involve both ventricles. Of the 50 patients, 28 (56%) had RV dysfunction, 4 with poorly defined PF status. Of the remaining 24 patients, 16 (67%) had lung disease, 8 (33%) had PH, and 10 (42%) had LV involvement. Ten patients had greater than one of these findings, and 4 had all 3. In contrast, in 4/24 patients (17%), RV dysfunction could not be explained by these mechanisms, despite severely reduced RV strain. In conclusion, RV dysfunction is common in patients with sarcoidosis and is usually associated with either direct LV involvement, lung disease, or PH, but may occur in the absence of these mechanisms, suggesting the possibility of isolated RV involvement and underscoring the need for imaging protocols that would include RV strain analysis.

Subclinical involvement of the heart and its associated factors in patients with sarcoidosis with normal systolic function using 2D speckle tracking

“…17 According to Patel et al study, 56% of patients had right ventricular dysfunction, and these changes could be associated with the absence of left ventricular dysfunction or even independent of pulmonary function impairment. 18 Schouver et al showed that the total left ventricular strain or LV GLS in the sarcoidosis patients was reduced compared to the control group, 19 which was consistent with our results. In their study, the LV GLS index was an important indicator in the evaluation of cardiac sarcoidosis.…”

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

“…Our systematic review identified 137 potentially relevant articles; the full text of 13 was reviewed and 9 were selected. None of the studies compared TTE to no testing; rather, all were nonrandomized studies that selected patients with sarcoidosis (most enrolled patients who had persistent respiratory symptoms despite treatment of their sarcoidosis) and reported the frequency of abnormal TTE results (180,184,(191)(192)(193)(194)(195)(196)(197). Some studies also reported the rate of confirmation by right heart catheterization.…”

A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and Inflammation & Infection Committees of the European Association of Nuclear Medicine, the European Association of Cardiovascular Imaging, and the American Society of Nuclear Cardiology

“…23 Further, a reduced global longitudinal strain is an independent predictor of adverse events among patients with suspected cardiac sarcoidosis. 25 The presence of LGE on CMR, including focal LGE, 76 and the extent of LGE (LGE mass C20% of LV mass), is associated with a higher risk of death or VT and a lower likelihood of improvement in LV function. 83,99 The prognostic value of other CMR findings have been less well studied and no reports are available for T2 weighted CMR.…”